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Pediatric Cardiology

, Volume 40, Issue 1, pp 168–176 | Cite as

Immediate Postnatal Ventricular Performance Is Associated with Mortality in Hypoplastic Left Heart Syndrome

  • Gabriel AltitEmail author
  • Shazia Bhombal
  • Valerie Y. Chock
  • Theresa A. Tacy
Original Article

Abstract

Right ventricular (RV) function as assessed by deformation has been evaluated prenatally and after palliation in hypoplastic left heart syndrome (HLHS). However, limited data exist about the immediate postnatal cardiac adaptation and RV function in HLHS. We compared echocardiographic measures of cardiac performance in HLHS versus controls in their first week of life. As a secondary objective, we evaluated if markers at the first echocardiogram were associated with mid- and long-term outcomes. Clinical and echocardiographic data of patients with HLHS between 2013 and 2016 were reviewed. The study population was matched with controls whose echocardiograms were obtained due to murmur or rule out coarctation. Speckle-tracking echocardiography was used to assess deformation. Thirty-four patients with HLHS and 28 controls were analyzed. Age at echocardiogram was similar between HLHS and controls. The RV of HLHS was compared to both RV and left ventricle (LV) of controls. HLHS deformation parameters [RV peak global longitudinal strain (GLS), global longitudinal strain rate (GLSR)] and tricuspid annular plane systolic excursion (TAPSE) were decreased compared to RV of controls. The LV-fractional area change, peak GLS, GLSR, circumferential strain, and strain rate of controls were higher than the RV of HLHS. Calculated cardiac output (CO) was higher in the HLHS group (592 vs. 183 mL/kg/min, p = 0.0001) but similar to the combined LV and RV output of controls. Later mortality or cardiac transplantation was associated with the RV CO and RV stroke distance at initial echocardiogram. Cox proportional hazard regression determined that restriction at atrial septum, decreased initial RV stroke distance and decreased TAPSE had a higher risk of death or cardiac transplantation. TAPSE and RV stroke distance by velocity time integral had adequate inter-reader variability by Bland–Altman plot and Pearson’s correlation. Our study found that the HLHS RV deformation is decreased in the early postnatal period when compared to both LV and RV of controls, but deformation was not associated with mid- and long-term outcomes. Later mortality or cardiac transplantation was associated with decreased initial stroke distance and cardiac output. Early evaluation of patients with HLHS should include an assessment of stroke distance and future research should evaluate its implication in management strategies.

Keywords

Hypoplastic left heart syndrome (HLHS) Speckle-tracking echocardiography Ventricular performance Cardiac output Neonatal adaptation Mortality Outcomes 

Abbreviations

AA

Aortic atresia

AS

Aortic stenosis

AV

Aortic valve

CO

Cardiac output

DICOM

Digital imaging and communications in medicine format

EF

Ejection fraction

ECMO

Extracorporeal membrane oxygenation

FAC

Fractional area change

HUS

Head ultrasound

HLHS

Hypoplastic left heart syndrome

IVH

Intra-ventricular hemorrhage

LV

Left ventricle

LVOT

Left ventricular outflow tract

MRI

Magnetic resonance imaging

MD

Minute distance

MA

Mitral atresia

MS

Mitral stenosis

MV

Mitral valve

NICU

Neonatal intensive care unit

pGLS

Peak global longitudinal systolic

PW

Pulsed-wave

PV

Pulmonary valve

RV

Right ventricle

SGA

Small for gestational age

SR

Strain rate

SVC

Superior vena cava

TAPVR

Total anomalous pulmonary venous return

TV

Tricuspid valve

TAPSE

Tricuspid annular plane systolic excursion

VVI

Velocity vector imaging

VTI

Velocity time integral

VSD

Ventricular septal defect

Notes

Compliance with Ethical Standards

Conflict of interest

We have no conflict of interest related to the content of this study. Gabriel Altit is the author that wrote the first draft. There was no payment, grant, or honorarium given to anyone to produce the manuscript. All the authors (Gabriel Altit, Shazia Bhombal, Valerie Y. Chock, and Theresa A. Tacy) declare that they have no conflict of interest.

Ethical Approval

This study was approved by the institutional review board of Stanford University (protocol—IRB-39544). All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Supplementary material

246_2018_1974_MOESM1_ESM.docx (13 kb)
Supplementary material 1 (DOCX 14 KB)

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.NeonatologyMcGill University - Montreal Children’s HospitalMontrealCanada
  2. 2.Department of Developmental and Neonatal MedicineStanford University – Lucile Packard Children’s HospitalPalo AltoUSA
  3. 3.Pediatric CardiologyStanford University – Lucile Packard Children’s HospitalPalo AltoUSA

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